Neuroanatomical localization of faciobrachial dystonic seizures in <scp>LGI1</scp> ‐antibody encephalitis

Faciobrachial dystonic seizures (FBDS), paroxysmal dizziness spells, and thermal sensory attacks are highly frequent and stereotypic phenomena experienced in leucine-rich glioma inactivated 1 (LGI1)-antibody encephalitis. This study aims to describe the electrophysiologic mechanism underlying these pathognomonic symptoms. LGI1-antibody encephalitis patients with active symptoms were enrolled from two separate centers in South Korea and the United States. Patients were evaluated with simultaneous magnetoencephalography (306 channels) and electroencephalography. Regional alterations in neuronal excitability represented by interictal epileptiform discharges were present in the faciobrachial area of the motor cortex, insula, and somatosensory cortex, somatotopically aligned with each of the ictal semiologies observed in patients. FBDS and other LGI1-antibody encephalitis-specific spells localized to cortical regions neuroanatomically corresponding to ictal semiologies: the faciobrachial homunculus (FBDS), insular cortex (paroxysmal dizziness spells), and somatosensory cortex (thermal sensory attacks). Our findings support the ictal hypothesis underlying these unique phenomena.

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Publication Details

Journal
Epilepsia
Published
2026-03-13
DOI
https://doi.org/10.1002/epi.70197
Primary Topic
Autoimmune Neurological Disorders and Treatments
Type
article
Field-Weighted Citation Impact
0.00
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article

Neuroanatomical localization of faciobrachial dystonic seizures in LGI1 ‐antibody encephalitis

Seung Bong Hong, Kon Chu, Samantha A. Banks, Kyung Min Kim et al.
Epilepsia
Autoimmune Neurological Disorders and Treatments
article

Neuroanatomical localization of faciobrachial dystonic seizures in LGI1 ‐antibody encephalitis

Seung Bong Hong, Kon Chu, Samantha A. Banks, Kyung Min Kim, Yoonhee Shin, Yihui Goh, Maryam Karaminiya, Soo Hyun Ahn, Sarosh Irani, Soon‐Tae Lee, Woo‐Seok Ha, Ki‐Young Jung, Sang‐Kun Lee, Yoonhyuk Jang
article en

Abstract

Faciobrachial dystonic seizures (FBDS), paroxysmal dizziness spells, and thermal sensory attacks are highly frequent and stereotypic phenomena experienced in leucine-rich glioma inactivated 1 (LGI1)-antibody encephalitis. This study aims to describe the electrophysiologic mechanism underlying these pathognomonic symptoms. LGI1-antibody encephalitis patients with active symptoms were enrolled from two separate centers in South Korea and the United States. Patients were evaluated with simultaneous magnetoencephalography (306 channels) and electroencephalography. Regional alterations in neuronal excitability represented by interictal epileptiform discharges were present in the faciobrachial area of the motor cortex, insula, and somatosensory cortex, somatotopically aligned with each of the ictal semiologies observed in patients. FBDS and other LGI1-antibody encephalitis-specific spells localized to cortical regions neuroanatomically corresponding to ictal semiologies: the faciobrachial homunculus (FBDS), insular cortex (paroxysmal dizziness spells), and somatosensory cortex (thermal sensory attacks). Our findings support the ictal hypothesis underlying these unique phenomena.

Epilepsia
Mayo Clinic (US), National University of Singapore (SG), Yonsei University (KR), Jacksonville College (US), Seoul National University Hospital (KR), The Seoul Institute (KR), St. Peter's Hospital (CA), Mayo Clinic in Florida (US), National University Health System (SG)
Openalex Percentile: Top 30%
Autoimmune Neurological Disorders and Treatments
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